Adrenal insufficiency pathophysiology
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:
Overview
The exact pathogenesis of [disease name] is not fully understood.
OR
It is thought that [disease name] is the result of / is mediated by / is produced by / is caused by either [hypothesis 1], [hypothesis 2], or [hypothesis 3].
OR
[Pathogen name] is usually transmitted via the [transmission route] route to the human host.
OR
Following transmission/ingestion, the [pathogen] uses the [entry site] to invade the [cell name] cell.
OR
[Disease or malignancy name] arises from [cell name]s, which are [cell type] cells that are normally involved in [function of cells].
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The progression to [disease name] usually involves the [molecular pathway].
OR
The pathophysiology of [disease/malignancy] depends on the histological subtype.
Pathophysiology
Physiology
Adrenal insufficiency arises due to the deficiency of adrenocortical hormones.The normal physiology of adrenocortical hormone production can be understood as follows: The adrenal cortex is divided into three zones from outside to inside as - Zona glomerulosa, zona fasciculata and zona reticularis which are responsible for the production of streroidogenic hormones like mineralocorticoid, glucocorticoid and androgens respectively. [1]
Pathogenesis
The pathophysiology of adrenal insufficiency depends on the etiological subtype.
Autoimmune adrenalitis: Both humoral and cell-mediated immune mechanisms attack enzymes involved in adrenocortical hormone synthesis. Autoantibodies against 21-hydroxylase, an enzyme required in the biosynthesis of adrenocortical hormones can be found. The patients remain asymptomatic until 90% of the cortex is destroyed. It can present as isolated autoimmune adrenalitis in 30-40% of cases or as an autoimmune polyglandular syndrome in 60-70% of cases. The autoimmune polyglandular syndrome in subclassified as autoimmune polyglandular syndrome type 1(APS type 1) and autoimmune polyglandular syndrome type 2 (APS type 2)
Genetics
Autoimmune adrenalitis: Strong genetic association has been seen with HLA DR3/DQ2 and DR4/DQ8.
- The autoimmune polyglandular syndrome type 1 is transmitted in an autosomal recessive pattern. Gene involved in the pathogenesis is the autoimmune regulator gene (AIRE).
- The autoimmune polyglandular syndrome type 2 shows a polygenic inheritance. A strong association has been shown with HLA DR3 of MHC.
[Disease name] is transmited in [mode of genetic transmission] pattern.
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Genes involved in the pathogenesis of [disease name] include:
- [Gene1]
- [Gene2]
- [Gene3]
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The development of [disease name] is the result of multiple genetic mutations such as:
- [Mutation 1]
- [Mutation 2]
- [Mutation 3]
Associated Conditions
- Autoimmune polyglandular syndrome type 1 is also known as Autoimmune Polyendocrinopathy-Candidiasis-Ectodermal Dystrophy (APECED). It is associated with chronic mucocutaneous candidiasis, hyperparathyroidism, total alopecia.
- Autoimmune polyglandular syndrome type 2 is associated with autoimmune thyroiditis, vitiligo, premature ovarian failure, type 1 diabetes mellitus, pernicious anemia.
Gross Pathology
On gross pathology, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].
Microscopic Pathology
On microscopic histopathological analysis, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].